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Utility of LRF/Pokemon and NOTCH1 protein expression in the distinction between nodular lymphocyte-predominant Hodgkin lymphoma and classical Hodgkin lymphoma.
[hodgkin lymphoma, classical]
Classical
Hodgkin
lymphoma
(
CHL
)
and
nodular
lymphocyte-predominant
Hodgkin
lymphoma
(
NLPHL
)
are
considered
separate
entities
with
different
prognosis
and
treatment
.
However
,
morphologic
features
can
be
similar
and
immunohistochemical
studies
are
essential
in
the
distinction
;
thus
,
determination
of
additional
biomarkers
is
of
utmost
importance
.
LRF
/
Pokemon
is
a
proto-oncogene
,
an
interacting
partner
co
-expressed
with
BCL
6
in
germinal
centers
and
highly
expressed
in
diffuse
large
B-
cell
lymphoma
and
follicular
lymphoma
.
Conversely
,
loss
of
the
LRF
gene
in
mouse
hematopoietic
stem
cells
results
in
complete
block
of
early
B
cell
development
with
concomitant
Notch
de
-repression
,
indicating
its
critical
role
in
B
versus
T
cell
fate
decision
at
the
hematopoietic
stem
cell
stage
.
For
the
first
time
,
we
show
that
LRF
/
Pokemon
is
predominantly
expressed
in
NLPHL
cases
as
is
BCL
6
with
low
to
absent
NOTCH
1
protein
expression
;
while
Hodgkin
Reed
-
Sternberg
(
HRS
)
cells
in
CHL
show
low
to
absent
BCL
6
and
LRF
/
Pokemon
expression
with
higher
NOTCH
1
expression
.
We
illustrate
a
potential
functional
interaction
between
LRF
and
BCL
6
in
NLPHL
pathogenesis
,
and
differential
expression
of
LRF
/
Pokemon
and
NOTCH
1
proteins
in
CHL
thus
showing
differential
expression
,
making
for
an
additional
diagnostic
marker
and
therapeutic
target
.
Diseases
Validation
Diseases presenting
"considered separate entities with different prognosis and treatment"
symptom
hodgkin lymphoma, classical
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